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PROCEEDINGS
OF THE 32ND BETHESDA CONFERENCE
CARE OF THE ADULT WITH CONGENITAL HEART DISEASE
JACC Vol. 37, 2001: 1161-98
32nd
Bethesda Conference:
Care of the Adult With Congenital Heart Disease
Gary D. Webb, MD, FACC, Conference Co-Chair, Roberta
G. Williams, MD, FACC, Conference Co-Chair
Introduction
Consider
the changes in the practice of cardiology in the lifetime
of a 40-year-old patient with tetralogy of Fallot. In
the 1960s it was common for adult and pediatric cardiologists
to practice together in a medical school setting, with
the exception of a few freestanding children's hospitals.
Patients were discussed at a joint conference. Without
the demands of highly technical procedures, there was
a greater commonality of knowledge and skills than we
find today. Infant surgery was practically non-existent,
and surgery on young children was usually confined to
palliative procedures. Repair of lesions such as tetralogy
of Fallot or atrial septal defect was generally delayed
until early adolescence. Patients who survived until
adult life were few.
The
rapid technological developments that have occurred
in the three past decades have enabled patients with
more complex congenital cardiac defects to survive into
adult life. Now, for the first time, the number of adults
with congenital heart disease (CHD) equals the number
of children with the disorder. The range of abnormalities
and the complexities of postoperative anatomy are now
well beyond the educational and experiential background
of the adult cardiologist. Pediatric cardiologists,
who often follow their patients into adult life, are
at a disadvantage when confronted by superimposed acquired
adult diseases. Both groups of subspecialists are drawn
deeper into the complexities of their own fields and
have vanishingly few opportunities to interact in an
ongoing professional forum. A few adult congenital heart
disease (ACHD) programs have served as referral centers
for adult patients who require surgical or catheter
intervention, but most patients do not have their cases
reviewed in these centers. There is no organized effort
at monitoring clinical outcomes for these patients,
and the frequency with which these patients appear late
at tertiary centers with avoidable complications indicates
that health care delivery to this population falls far
short of that for adults with acquired heart disease
or children with CHD.
The
transition from childhood to adulthood is particularly
difficult for patients with chronic disease. Some are
multiply-handicapped with cognitive and physical disabilities.
The less disabled do not qualify for public insurance
and are at risk for the development of secondary disability
as a result of inadequate continuity of care. Adolescence
is a time of particular anxiety about conformity, social
success, and uncertainty about the future. Denial is
a frequently used defense, coupled with a sense of immortality
and a desire for risk taking. Relocation for educational
or occupational reasons is common and often results
in the transfer of inadequate information to the new
provider. When one is distracted by the demands of job
and family, it is easier to put aside routine health
care, particularly when one is asymptomatic. Considering
the human and financial resources that have been expended
in nurturing these individuals to adult age, it is important
to improve the system of care for adults with CHD in
order for them to maintain the functional status that
has been so hard won.
The
American College of Cardiology is the professional organization
where adult cardiovascular specialists, pediatric cardiologists,
and cardiothoracic surgeons join together in the interests
of care delivery, professional education, and advocacy.
The authors are grateful for the opportunity to bring
together these formidable resources in the format of
the Bethesda Conference. The conference was organized
into five sections that represent: 1) the present number
of adults with CHD, 2) the special needs of these patients,
3) the workforce needed to deliver ACHD health care,
4) recommendations for a system of health care delivery
that would produce the best clinical outcomes with the
most efficient use of resources, and 5) the steps needed
to ensure access to adequate health care for these patients.
The answers reduce to three requirements: 1) education
of a modest number of ACHD specialists who can lead
highly specialized teams and serve as a referral and
continuing educational resource for the greater number
of providers with training in either adult or pediatric
cardiology; 2) integration of highly specialized centers
with community-based providers and with each other in
order to provide a high level of care for all patients
and to provide an informational base for continual improvement
in care; and 3) unfettered access to an appropriately
trained provider for all adults with CHD. These actions
would require a modest outlay of resources, but the
status quo will result in greater long-term expenditures
for terminal care and loss of productivity for this
ever-growing population. The product of this conference
is a comprehensive analysis of the problem and a recommendation
for corrective steps. The realization of the vision
articulated in the following sections will require the
passion and constancy of focus that have been exhibited
by so many of the participants in this conference.
© 2001 by The American College of
Cardiology
Published by Elsevier
Science Inc.
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